Abstract:
:The 2013 discovery of calreticulin (CALR) mutations in myeloproliferative neoplasms was attended by their association with longer survival in primary myelofibrosis (PMF). Subsequent studies have suggested prognostic distinction between type 1/like and type 2/like CALR mutations and detrimental effect from triple-negative mutational status. Among 709 Mayo Clinic patients with PMF, 467 (66%) harbored JAK2, 112 (16%) CALR type 1/like, 24 (3.4%) CALR type 2/like, 38 (5.4%) MPL mutations and 68 (10%) were triple-negative. Survival was longer with type 1/like CALR, compared to JAK2 (HR 2.6, 95% CI 1.9-3.5), type 2/like CALR (HR 2.5, 95% CI 1.4-4.5), MPL (HR 1.8, 95% CI 1.1-2.9) and triple-negative mutational status (HR 2.4, 95% CI 1.6-3.6), but otherwise similar between the non-type 1/like CALR mutational states (P = .41). In multivariable analysis, the absence of type 1/like CALR (P < .001; HR 2, 95% CI 1.4-2.7), presence of ASXL1/SRSF2 mutations (P < .001; HR 1.9, 95% CI 1.5-2.4) and DIPSS-plus (P < .001) were each predictive of inferior survival. Furthermore, among 210 patients with ASXL1/SRSF2 mutations, survival was significantly longer in the presence vs. absence of type 1/like CALR mutations (median 5.8 vs. 2.9 years; P < .001). Triple-negative status did not disclose additional prognostic information for overall or leukemia-free survival. The observations regarding the prognostic distinction between CALR mutation variants were validated in an external cohort of 386 patients from the University of Florence Careggi hospital. We conclude that type 1/like CALR mutations in PMF not only predict superior survival, but also partially amend the detrimental effect of high molecular risk mutations.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Tefferi A,Nicolosi M,Mudireddy M,Szuber N,Finke CM,Lasho TL,Hanson CA,Ketterling RP,Pardanani A,Gangat N,Mannarelli C,Fanelli T,Guglielmelli P,Vannucchi AMdoi
10.1002/ajh.24978subject
Has Abstractpub_date
2018-03-01 00:00:00pages
348-355issue
3eissn
0361-8609issn
1096-8652journal_volume
93pub_type
杂志文章abstract::To better assess the efficacy and safety of rituximab in adults' warm antibody autoimmune hemolytic anemia (wAIHA), we conducted a retrospective study including 27 adults (mean age 49.7 +/- 21 years) with either primary (n = 17) or secondary (n = 10) wAIHA. On average, the patients received 2.1 +/- 1.4 treatment lines...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.21341
更新日期:2009-03-01 00:00:00
abstract::We aimed to determine the survival benefits of chemotherapy (CT) added to radiotherapy (RT) in different risk groups of patients with early-stage extranodal nasal-type NK/T-cell lymphoma (ENKTCL), and to investigate the risk of postponing RT based on induction CT responses. A total of 1360 patients who received RT wit...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ajh.25878
更新日期:2020-09-01 00:00:00
abstract::Immune thrombocytopenia is frequently encountered in medical practice and is generally accepted as being caused by an IgG antibody. The capability of detecting platelet-bound IgG as a diagnostic and therapeutic modality is critical for appropriate care and management of patients with idiopathic thrombocytopenic purpur...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830090402
更新日期:1980-01-01 00:00:00
abstract::Thrombotic thrombocytopenic purpura (TTP) is a microangiopathy syndrome caused by a congenital or acquired deficiency of ADAMTS13, a plasma metalloprotease that cleaves von Willebrand factor (VWF) and thus prevents the formation of platelet-rich thrombi in the microcirculation. TTP can be fatal if not appropriately an...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23527
更新日期:2013-10-01 00:00:00
abstract::Because of the T-cell abnormalities observed in Hodgkin's disease and the growing number of Hodgkin's disease cases observed in association with the acquired immunodeficiency syndrome (AIDS), concern has been expressed that a retrovirus may be the primary cause of Hodgkin's disease. We examined plasma specimens from 1...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830270104
更新日期:1988-01-01 00:00:00
abstract::Graft failure is a life-threatening complication after allogeneic hematopoietic stem cell transplantation (HSCT). Graft failure is more prevalent after umbilical cord blood transplantation (UCBT) compared with conventional adult stem cell sources. We identified 21 consecutive patients who experienced graft failure aft...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23845
更新日期:2014-12-01 00:00:00
abstract::Four factor XI (F XI)-deficient patients are described, all of whom formed circulating anticoagulants against F X1. In the three most severely affected patients (F XI 0%-6% activity), the anticoagulant appeared to have been stimulated by plasma infusion. However, in the milder case (25% F XI activity), no infusion had...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830260405
更新日期:1987-12-01 00:00:00
abstract::Autologous 51Cr-platelet kinetic studies were performed in splenectomized mongrel dogs. Mean survival time of PRP-platelets was 5.4 +/- 1.5 (SD) days (n = 6). The curves, though slightly curvilinear, showed mostly a linear type of decay, denoting that platelet removal from the circulation is mainly determined by aging...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830170407
更新日期:1984-01-01 00:00:00
abstract::Severe chronic neutropenia (SCN) is defined as an absolute neutrophil (ANC) of less than 0.5 x 10(9)/L, lasting for months or years. Congenital, cyclic, and idiopathic neutropenia are principal categories of SCN. Since 1994, the Severe Chronic Neutropenia International Registry (SCNIR) has collected data to monitor th...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.10255
更新日期:2003-02-01 00:00:00
abstract::Chimeric antigen receptors (CARs) can be introduced into T-cells redirecting them to target specific tumor antigens. CAR-modified T cells targeting CD19 have shown remarkable activity against CD19+ malignancies including B cell acute lymphocytic leukemia (ALL), chronic lymphocytic leukemia (CLL), and non-Hodgkin lymph...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.24238
更新日期:2016-01-01 00:00:00
abstract::We present a case of chronic tonsillitis in a patient with chronic lymphocytic leukemia. Despite empiric radiation and antibiotic therapy, the patient's sore throat and tonsillar enlargement persisted. Excisional biopsy of the involved tonsil revealed the presence of Cryptococcus neoformans. Blood cultures also yielde...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830250413
更新日期:1987-08-01 00:00:00
abstract::The improved microchromatographic procedure for the detection of Hb-S and/or Hb-C in cord blood at birth (Schroeder et al.: J. Lab Clin Med 86:528-532, 1975) as well as a modification thereof may also be used for the quantitative determination of Hb-F in the presence of Hb-S and/or Hb-C. However, Hb-A interferes and m...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830010308
更新日期:1976-01-01 00:00:00
abstract::We aimed to comprehensively study sex differences in disease and patients' characteristics, treatment and outcomes in patients with follicular lymphoma (FL) in the United States (USA) utilizing the National LymphoCare Study registry (2004-2014). Among evaluable males (n = 1277) and females (n = 1375) with FL, females ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24401
更新日期:2016-08-01 00:00:00
abstract::We describe two patients with mild hemophilia A (MHA) who developed high titer inhibitor (HTI) following intensive recombinant factor VIII (rFVIII) concentrate replacement for surgery and trauma. Intranasal desmopressin was instituted shortly following immunosuppressive therapy (IST) and activated prothrombin complex ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.1176
更新日期:2001-11-01 00:00:00
abstract::FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia (F/P+ MN-eo) is a rare disease: robust epidemiological data are lacking and reported issues are scarce, of low sample-size and limited follow-up. Imatinib mesylate (IM) is highly efficient but no predictive factor of relapse after discontinuation has yet been i...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ajh.25945
更新日期:2020-11-01 00:00:00
abstract:DISEASE OVERVIEW:Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 9,000 new patients annually and representing approximately 11% of all lymphomas in the United States. DIAGNOSIS:HL is composed of two distinct disease entities; the more commonly diagnosed classical HL and the rare nodular lymph...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23348
更新日期:2012-12-01 00:00:00
abstract::Bone marrow cells, peripheral blood lymphocytes, and sera from patients with sickle-cell anemia in hypoproliferative crisis were studied in the plasma clot culture system in the presence or absence of erythropoietin (Epo). Bone marrow cells from five patients demonstrated a marked ability to form erythroid colonies in...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830080103
更新日期:1980-01-01 00:00:00
abstract::Intravenous immunoglobulin (IVIG) therapy is associated with rare reports of thromboembolic events and severe hyponatremia. We hypothesized that IVIG therapy may result in hyperproteinemia, increased serum viscosity, and pseudohyponatremia. We conducted a prospective observational study to evaluate the incidence of hy...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10325
更新日期:2003-06-01 00:00:00
abstract::Among 47 patients with thrombotic thrombocytopenic purpura (TTP), 8 patients were diagnosed to have postoperative-TTP. Two patients underwent vascular surgery, 5 patients coronary artery bypass grafts, and 1 patient resection of myocardial sarcoma. Prior to surgery, all patients except one had normal hemograms and pla...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199609)53:1<11::AID-AJH3>3
更新日期:1996-09-01 00:00:00
abstract::Hydroxyurea (HU) is a widely used cytotoxic agent that is known to induce fetal hemoglobin (HbF) production and is presently used to ameliorate the severity of pain episodes in patients with sickle cell anemia (HbSS). Previously we have shown that HU inhibits growth of burst forming unit-erythroid (BFU-E) colonies in ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200011)65:3<227::aid-ajh9>3.0.co
更新日期:2000-11-01 00:00:00
abstract::Unprecedented advances in our understanding of the pathobiology, prognostication, and therapeutic options in mantle cell lymphoma (MCL) have taken place in the last few years. Heterogeneity in the clinical course of MCL-indolent vs aggressive-is further delineated by a correlation with the mutational status of the var...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25487
更新日期:2019-06-01 00:00:00
abstract::The FAB classification of myelodysplastic syndromes (MDS) has been useful in predicting prognosis; however, additional methods are required to detect patients at high risk for early conversion to acute nonlymphoblastic leukemia (ANLL). Using a panel of monoclonal antibodies to myelomonocytic surface antigens (MMSA) an...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830430302
更新日期:1993-07-01 00:00:00
abstract::The aim of this study was to investigate whether genetic polymorphisms in cytochrome P450s (CYPs), glutathione S-transferases (GSTs), and N-acetyltransferases (NATs) genes modify the relationship between alcohol consumption and risk of non-Hodgkin's lymphoma (NHL) in a population-based, case-control study including 1,...
journal_title:American journal of hematology
pub_type: 信件
doi:10.1002/ajh.21608
更新日期:2010-03-01 00:00:00
abstract::Measurements of the iron regulatory hormone hepcidin by various methodologies and laboratories are not harmonized. As a result different numeric results are obtained for the same clinical sample. We investigated whether better agreement between plasma hepcidin methods can be achieved by harmonization. Native plasma po...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23289
更新日期:2012-10-01 00:00:00
abstract::Increasing dose intensity (DI) of chemotherapy for patients with aggressive non-Hodgkin lymphoma (NHL) may improve outcomes at the cost of increased toxicity. This issue was addressed in a randomized trial aiming to double the DI of myelosuppressive drugs. Between 1994 and 1999, 250 patients with previously untreated ...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1002/ajh.23684
更新日期:2014-05-01 00:00:00
abstract::Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimisti...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.24235
更新日期:2016-01-01 00:00:00
abstract::Some properties of a monoclonal antibody generated against the fibrinogen component of a factor VIII preparation were investigated. The antibody bound with equal affinity in solid phase radioimmunoassays to fibrinogens isolated from both normal patients and patients with von Willebrand disease. It reacted in a sensiti...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830180202
更新日期:1985-02-01 00:00:00
abstract::The role of reduced-intensity conditioning (RIC) in adult acute lymphoblastic leukemia (ALL) remains unclear because of the small sample size, short follow-up duration, various regimens for conditioning and graft-versus-host disease (GVHD) prophylaxis, and the heterogeneity of selection criteria for transplantation. W...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.23465
更新日期:2013-08-01 00:00:00
abstract::Patients initially diagnosed with type 1 von Willebrand disease (VWD) have been reclassified as type 2 after a more exhaustive analysis in several studies. Our study's objectives were (1) to reanalyze patients that were previously diagnosed as type 1 to achieve a more accurate diagnosis and (2) to compare the von Will...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20470
更新日期:2005-11-01 00:00:00
abstract::A 69-year-old man with immunoblastic lymphadenopathy and autoimmune hemolytic anemia who had no previous exposure to blood products developed a severe febrile nonhemolytic transfusion reaction following the initial infusion of packed red blood cells. The reaction recurred with transfusion of packed red blood cells, bu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830140112
更新日期:1983-02-01 00:00:00